On respiratory function testing, the degree of gas transfer reduction is reflected by the decrease in the diffusing capacity of the lungs for carbon monoxide, which is also reduced in DM-IP. However, in patients with severe DM-A/SIP, accurate pulmonary function testing is impossible due to complications such as dyspnea and pneumomediastinum. Therefore, the PO2 is simple and prognostically useful. In the DM-A/SIP patients treated by early CSA/PDN combination therapy, ferritin $600 ng/ml and PO2 $45 Torr were independent prognostic factors, and all patients with both of these factors died. In these patients, the outcome could not be improved even by additional treatments such as IVCY and IVIG. Recently, mycophenolate mofetil and blood purification therapy have been reported to be effective in patients with refractory DM-A/SIP. In patients with a poor prognosis, immunosuppressant therapy such as IVCY and mycophenolate mofetil and blood purification therapy should be considered from the beginning of treatment in addition to early CSA/PDN combination therapy. The clinical course of DM-A/SIP is rapidly progressive and this biomarker may not reflect the disease activity in rapid course of interstitial pneumonia. This study was carried out retrospectively in a small number of patients. In addition, autoantibodies and lung high-resolution computed tomography findings were not evaluated in all enrolled patients or in a large number of patients. Prognostic factors associated with the disease must still be extracted by evaluating a larger number of patients. In mammalian organisms, Campesterol steroid hormones are indispensable for a normal development. Considering their action, these steroids are classified into three main groups. Mineralocorticoids, with aldosterone as the most important representative, regulate the salt household and hence the blood pressure. Cortisol belongs to the glucocorticoids and provides the organism with energy stimulating the gluconeogenesis. Finally, the sexual hormones, with androgens and estrogens, are fundamental for the formation of the sexual characteristics and the estrous cycle. Biosynthesis of all steroid hormones is initiated by CYP11A1 with the side chain cleavage of cholesterol yielding pregnenolone. This ratelimiting step of the steroid hormone biosynthesis is carried out in the inner mitochondrial membrane and displays three hydroxylation reactions. In the first step, carbon C22 of cholesterol is hydroxylated followed by an oxidative attack at C20, forming the intermediate 20,22-dihdroxycholesterol. The cleavage of the side chain of cholesterol is initiated by a third hydroxylation. The electrons necessary oxygen activation and substrate hydroxylation are transferred from NADH via a NADH-depending 14alpha-hydroxy-Sprengerinin-C ferredoxin reductase, adrenodoxin reductase, and a ferredoxin, adrenodoxin. The product pregnenolone serves as precursor for mineralocorticoids, glucocorticoids, as well as DHEA and its derived sexual hormones. To induce their biological activities, according to recent hypothesis steroid hormones have to be available in an unconjugated form to interact with their corresponding receptors. Conjugated steroids with sulfate groups have been regarded for a long time to be exclusively designated for excretion.